Hemoptysis as an initial manifestation of antiphospholipid antibody syndrome

Abstract

Antiphospholipid antibody syndrome (APS) is a multisystemic autoimmune disease that is characterized by thromboembolic events and/or obstetric morbidity in the presence of antiphospholipid antibodies. The most frequent pulmonary manifestations are pulmonary thromboembolism and pulmonary arterial hypertension. Recently, some rare cases have been described in which diffuse alveolar hemorrhage (DAH) appears as the initial manifestation of APS. We report the case of a 44-year-old female patient who presented to the ER with mild hemoptysis in the last month. Chest X-ray showed bilateral cotton-like infiltrates. Chest CT scan with contrast revealed several areas of densification of the lung parenchyma. Bronchoscopy with bronchoalveolar lavage was performed. In the autoimmunity study, positive anti-cardiolipin, anti-b2 glycoprotein antibodies and lupus anticoagulant were identified. The diagnosis of APS with DAH as form of presentation was established. The patient started treatment with prednisolone, mycophenolate mofetil, and hydroxychloroquine. In the following months, the patient had 2 additional hospitalizations due to recurrence of hemoptysis; therefore, rituximab was associated to the therapeutic scheme. DAH is a rare and life-threatening complication of APS and is associated with high mortality risk. Corticosteroid therapy induces remission in most patients; however, recurrence may occur in up to half of cases, which requires the introduction of a corticosteroid-sparing immunosuppressant.

Keywords

• diffuse alveolar hemorrhage
• antiphospholipid antibody syndrome
• corticosteroid therapy
• anti-tnf alpha